Dermatitis Herpetiformis- its Presentation and Management

Book Chapter: in Blistering Diseases: Clinical Features, Pathogenesis, Treatment, 2015

Dermatitis herpetiformis (DH) is an immune-mediated vesicular disease associated with gluten sensitivity, which has been reviewed in more detail in Chapter….. It is most common in individuals of North European descent, with prevalence rates varying between 1.2 and 75.3 per 100,000 people . Intensely pruritic papules and vesicles associated with excoriations are the characteristic cutaneous manifestations. Lesions are often grouped together, and predominantly affect extensor surfaces of the body, especially the elbows, dorsal forearms, knees, scalp, back, and buttocks . Histological features vary depending on the stage of disease, however, a neutrophilic infiltrate is invariably found within dermal papillae. Within 72 hours, subepidermal vesiculations develop at the papillary tips, which eventually connect to form larger subepidermal blisters that contain a mixed inflammatory infiltrate . Direct immunofluorescence (DIF) analysis from perilesional skin is considered the gold standard investigation for diagnosis of DH . The characteristic finding on DIF is the presence of granular deposits of IgA at the dermo-epidermal junction, sometimes with accompanying IgM and C3 deposits . Epidermal transglutaminaze (eTG) or transglutamaze 3 (TG3) has been identified as the target autoantigen in DH [17, 18] Serology should not be used as the primary investigation due to poor sensitivity (70%); but it might have an adjunctive role in the minority (<10% # 31) of cases where DIF is negative or equivocal. Up to 90% of DH patients have an associated gluten sensitive enteropathy (GSE) , and dietary control is an essential arm of management of DH . DH is a lifelong condition with a variable and often unpredictable clinical course, although a minority (10%) of patients might undergo spontaneous remission .

Journal of the American Academy of Dermatology, 2010

Journal of the European Academy of Dermatology and Venereology, 2021

Introduction Dermatitis herpetiformis (DH) is a chronic, pruritic, gluten-induced skin disorder characterized by subepidermal granular IgA deposition and a variable degree of enteropathy identical to that seen in coeliac disease. So far, there has been no European consensus about the management of DH.

Clinical, Cosmetic and Investigational Dermatology, 2015

Dermatitis herpetiformis (DH) is an inflammatory cutaneous disease with a chronic relapsing course, pruritic polymorphic lesions, and typical histopathological and immunopathological findings. According to several evidences, DH is considered the specific cutaneous manifestation of celiac disease, and the most recent guidelines of celiac disease have stated that, in celiac patients with a proven DH, a duodenal biopsy is unnecessary for the diagnosis. In this review, the most recent data about the diagnosis and the management of DH have been reported and discussed. In particular, in patients with clinical and/or histopathological findings suggestive for DH, the finding of granular IgA deposits along the dermal-epidermal junction or at the papillary tips by direct immunofluorescence (DIF) assay, together with positive results for anti-tissue transglutaminase antibody testing, allows the diagnosis. Thereafter, a gluten-free diet should be started in association with drugs, such as dapsone, that are able to control the skin manifestations during the first phases of the diet. In conclusion, although DH is a rare autoimmune disease with specific immunopathological alterations at the skin level, its importance goes beyond the skin itself and may have a big impact on the general health status and the quality of life of the patients.

Journal of the European Academy of Dermatology and Venereology, 2009

Dermatitis herpetiformis is a rare disease that should be considered the cutaneous expression of a gluten-sensitive enteropathy indistinguishable from celiac disease. Dermatitis herpetiformis is often misdiagnosed and to date no guidelines for the management of dermatitis herpetiformis have been published in Literature. The present guidelines have been prepared for dermatologists by the Group for Cutaneous Immunopathology of the Italian Society of Dermatology and Venereology. They reflect the best data available at the time of preparation and the clinical experience of the authors and the members of the Italian Group for Cutaneous Immunopathology. The diagnosis of dermatitis herpetiformis is established clinically, histologically, immunopathologically and serologically. A gluten-free diet (GFD) is the treatment of choice for patients with dermatitis herpetiformis. Dapsone and/or other drugs should be used during the period until the GFD is effective. In conclusion, the present guidelines provide evidence-based guidance for the diagnosis and treatment of dermatitis herpetiformis.

2021

A 44-year-old female teacher was referred to the Nutrition Consultation for Dermatology for a gluten-free diet. She had experienced a cluster of red and intensely itchy blisters at the cutaneous level for approximately six months. After a study by Dermatology, a herpetiform dermatitis was diagnosed. While waiting for the Nutrition Consultation, she removed the gluten from her diet and saw improvements in symptoms. Awaiting Gastroenterology Consultation.

Clinics in Dermatology, 1991

Canadian family physician Médecin de famille canadien, 2012

This article has been peer reviewed. Cet article a fait l'objet d'une révision par des pairs.

Frontiers in Immunology

Dermatitis herpetiformis (DH) is an inflammatory disease of the skin, considered the specific cutaneous manifestation of celiac disease (CD). Both DH and CD occur in gluten-sensitive individuals, share the same Human Leukocyte Antigen (HLA) haplotypes (DQ2 and DQ8), and improve following the administration of a gluten-free diet. Moreover, almost all DH patients show typical CD alterations at the small bowel biopsy, ranging from villous atrophy to augmented presence of intraepithelial lymphocytes, as well as the generation of circulating autoantibodies against tissue transglutaminase (tTG). Clinically, DH presents with polymorphic lesions, including papules, vesicles, and small blisters, symmetrically distributed in typical anatomical sites including the extensor aspects of the limbs, the elbows, the sacral regions, and the buttocks. Intense pruritus is almost the rule. However, many atypical presentations of DH have also been reported. Moreover, recent evidence suggested that DH is changing. Firstly, some studies reported a reduced incidence of DH, probably due to early recognition of CD, so that there is not enough time for DH to develop. Moreover, data from Japanese literature highlighted the absence of intestinal involvement as well as of the typical serological markers of CD (i.e., anti-tTG antibodies) in Japanese patients with DH. Similar cases may also occur in Caucasian patients, complicating DH diagnosis. The latter relies on the combination of clinical, histopathologic, and immunopathologic findings. Detecting granular IgA deposits at the dermal-epidermal junction by direct immunofluorescence (DIF) from perilesional skin represents the most specific diagnostic tool. Further, assessing serum titers of autoantibodies against epidermal transglutaminase (eTG), the supposed autoantigen of DH, may also serve as a clue for the diagnosis. However, a study from our group has recently demonstrated that granular IgA deposits may also occur in celiac patients with non-DH inflammatory skin diseases, raising questions about the effective role of eTG IgA autoantibodies in DH and suggesting the need of revising diagnostic criteria, conceivably emphasizing clinical aspects of the disease along with DIF. DH usually responds to the gluten-free diet. Topical clobetasol ointment or dapsone may be also applied to favor rapid disease control. Our review will focus on novel pathogenic insights, controversies, and management aspects of DH.

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